Update in understanding common variable immunodeficiency disorders (CVIDs) and the management of patients with these conditions.

Update in understanding common variable immunodeficiency disorders (CVIDs) and the management of patients with these conditions.
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DOI:
10.1111/j.1365-2141.2009.07669.x
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发表时间:
2009-06
影响因子:
6.5
通讯作者:
Cunningham-Rundles C
Cunningham-Rundles C
中科院分区:
医学2区
文献类型:
--
作者:
Chapel H;Cunningham-Rundles C

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常见变异性免疫缺陷病是一组由免疫球蛋白产生不足和一抗失败相关的异质性疾病。这种变异性导致难以对免疫发病机制或文献中报道的各种遗传异常的作用产生连贯的理解。最近尝试整理这些疾病的各种并发症并定义特定的临床表型,提高了我们对这些疾病的了解。一旦得到其他系列研究的完善和证实,这些研究将有助于提高预后的准确性和更好地管理临床并发症;它们还可以提供一种分析与新的免疫病理学和遗传学发现相关的结果的方法。
Common Variable Immunodeficiency Disorders are a mixed group of heterogeneous conditions linked by lack of immune globulin production and primary antibody failure. This variability results in difficulty in making coherent sense of either immunopathogenesis or the role of various genetic abnormalities reported in the literature. The recent attempt to collate the varied complications in these conditions and define particular clinical phenotypes, has improved our understanding of these diseases. Once refined and confirmed by studies in other series, these studies will facilitate improved accuracy of prognosis and better management of clinical complications; they may also provie a method of analysing outcomes as related to new immunopathologic and genetic findings.
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发表时间: 2005-08-01
期刊: NATURE GENETICS
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